AA amyloidosis secondary to adult onset Still's disease: About 19 cases

Delplanque, Marion; Pouchot, J.; Ducharme-Benard, Stéphanie; Fautrel, Bruno; Benyamine, A.; Daniel, Laurent; Gendre, Thierry; Grateau, Gilles; Georgin‐Lavialle, Sophie

doi:10.1016/j.semarthrit.2019.08.005

Cited by 15 publications

(12 citation statements)

References 86 publications

(64 reference statements)

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“…Due to the broad disturbance of cytokine signaling, AID can affect various organs and are thus associated with a high disease burden and severe physical, but also socioeconomic limitations ( 12 ). Furthermore, AID patients with persistent inflammation have a high risk of developing AA amyloidosis ( 13 , 14 ).…”

Section: Introductionmentioning

confidence: 99%

Effectiveness and Safety of JAK Inhibitors in Autoinflammatory Diseases: A Systematic Review

et al. 2022

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IntroductionAutoinflammatory diseases (AID) are rare diseases presenting with episodes of sterile inflammation. These involve multiple organs and can cause both acute organ damage and serious long-term effects, like amyloidosis. Disease-specific anti-inflammatory therapeutic strategies are established for some AID. However, their clinical course frequently includes relapsing, uncontrolled conditions. Therefore, new therapeutic approaches are needed. Janus Kinase inhibitors (JAKi) block key cytokines of AID pathogenesis and can be a potential option.MethodsA systematic review of the literature in accordance with the PRISMA guidelines was conducted. Three databases (MEDLINE, Embase and Cochrane Central Register of Controlled Trials) were searched for publications regarding the use of JAKi for AID. Data from the included publications was extracted and a narrative synthesis was performed. Criteria for defining treatment response were defined and applied.ResultsWe report data from 38 publications with a total of 101 patients describing the effects of JAKi in AID. Data on Type I Interferonopathies, Adult-Onset Still's Disease (AOSD), Systemic Juvenile Idiopathic Arthritis (sJIA), Familial Mediterranean Fever (FMF), and Behçet's Syndrome (BS) was identified. From a total of 52 patients with type I interferonopathies, in seven patients (7/52, 13.5%) a complete response was achieved, most (35/52, 67.3%) showed a partial response and a minority (10/52, 19.2%) showed no treatment response. For AOSD, a complete or a partial response was achieved by eleven (11/26, 42.3%) patients each. Two sJIA patients achieved complete response (2/4, 50%) and in two cases (2/4, 50%) a partial response was reported. Half of FMF patients showed a complete response and the other half had a partial one (3/6, 50.0%). Amongst BS patients most achieved a partial response (8/13, 61.5%). Five patients showed no response to therapy (5/13, 38.5%). Overall, the most frequent AEs were upper respiratory tract infections (17), pneumonia (10), BK virus viremia (10) and viruria (4), herpes zoster infection (5), viral gastroenteritis (2) and other infections (4).ConclusionThe results from this systematic review show that JAKi can be beneficial in certain AID. The risk of AEs, especially viral infections, should be considered. To accurately assess the risk benefit ratio of JAKi for AID, clinical trials should be conducted.

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Section: Introductionmentioning

confidence: 99%

Effectiveness and Safety of JAK Inhibitors in Autoinflammatory Diseases: A Systematic Review

et al. 2022

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“…We found eight (2.8%) decedents with an amyloidosis notification either as the UCD or an NUCD. Recently, a systematic review of the literature (1971–2018) estimated the prevalence of AA amyloidosis in AOSD to be 0.88% [ 40 ]. In this article, Delplanque et al described 19 patients with a case fatality rate of 21%.…”

Section: Discussionmentioning

confidence: 99%

Still’s Disease Mortality Trends in France, 1979–2016: A Multiple-Cause-of-Death Study

Borciuch

Fauvernier

Gerfaud‐Valentin

et al. 2021

JCM

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Still’s disease (SD) is often considered a benign disease, with low mortality rates. However, few studies have investigated SD mortality and its causes and most of these have been single-center cohort studies. We sought to examine mortality rates and causes of death among French decedents with SD. We performed a multiple-cause-of-death analysis on data collected between 1979 and 2016 by the French Epidemiological Center for the Medical Causes of Death. SD-related mortality rates were calculated and compared with the general population (observed/expected ratios, O/E). A total of 289 death certificates mentioned SD as the underlying cause of death (UCD) (n = 154) or as a non-underlying causes of death (NUCD) (n = 135). Over the study period, the mean age at death was 55.3 years (vs. 75.5 years in the general population), with differences depending on the period analyzed. The age-standardized mortality rate was 0.13/million person-years and was not different between men and women. When SD was the UCD, the most frequent associated causes were cardiovascular diseases (n = 29, 18.8%), infections (n = 25, 16.2%), and blood disorders (n = 11, 7.1%), including six cases (54%) with macrophage activation syndrome. As compared to the general population, SD decedents aged <45 years were more likely to die from a cardiovascular event (O/E = 3.41, p < 0.01); decedents at all ages were more likely to die from infection (O/E = 7.96–13.02, p < 0.001).

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“…По мнению авторов, низкая частота АА-амилоидоза могла также объясняться своевременно начатой терапией. При болезни Стилла взрослых АА-амилоидоз является достаточно редким осложнением и встречается не более, чем у 1% больных [70]. Как правило, развитие АА-амилоидоза отмечается в возрасте 3b-компо 5-40 лет у пациентов с длительным течением заболевания, плохо контролируемой активностью заболевания и деструктивным поражением суставов.…”

Section: аа-амилоид амилоидоз при аутовоспалительных заболеванияхunclassified

AA-amyloidosis in autoinflammatory diseases

Рамеев¹,

Moiseev²,

Lysenko³

2021

CPT

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AA amyloidosis complicates various chronic inflammatory disorders and is characterized by the accumulation of amyloid fibrils composed of serum amyloid A protein, an acute phase reactant. In recent decades, the role of chronic infections and rheumatoid arthritis in the ethiology of AA amyloidosis have decreased significantly as a result of their treatment improvement, whereas both monogenic (familial Meditarranean fever, cryopirin-associated periodic syndrome, etc.) or polygenic (ankylosing spondilitis, psoriatic arthritis, adult onset Still’s disease, etc) autoinflammatory diseases more frequently account for AA-amyloidosis today. Autoinflammatory diseases are a consequence of innate immunity disorders although the latter can contribute to the pathogenesis of autoimmune diseases as well. In patients with autoinflammatory diseases, the suppression of inflammation, even subclinical, is essential to prevent development or progression of AA amyloidosis. The choice of inflammatory agents that can be used to achieve this aim depends on the pathogenesis of autoinflammation, e.g. key mediators that are involved in the activation of inflammatory cascade.

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AA amyloidosis secondary to adult onset Still's disease: About 19 cases

Cited by 15 publications

References 86 publications

Effectiveness and Safety of JAK Inhibitors in Autoinflammatory Diseases: A Systematic Review

Effectiveness and Safety of JAK Inhibitors in Autoinflammatory Diseases: A Systematic Review

Still’s Disease Mortality Trends in France, 1979–2016: A Multiple-Cause-of-Death Study

AA-amyloidosis in autoinflammatory diseases

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